Juvenile gout.

The rarity of gout in children prompts the following report of two cases, both apparently 'primary' in nature. On occasion juvenile gout may result from a demonstrable cause, falling into the 'secondary' category. In secondary gout both blood and urinary levels of uric acid tend to be higher than in primary gout, and the ratio of renal clearance of uric acid to glomerular filtration rate is usually increased. The miscible pool of uric acid and its rate of turnover are also increased. Gout and aleukaemic leukaemia in a boy aged 5 years was described by Vining and Thomson (1934), this being the first description of the association of gout and leukaemia in a child. It is of interest that there was a positive family history of gout in this case. Kersley, Mandel, and Jeffrey (1950) reported the case of a male who suffered the onset of gout when aged 18 years and died aged 23. Talbott (1959) considers this case also to be one of myeloid metaplasia. Other blood disorders reported as causing juvenile gout include congenital haemolytic anaemia (Lambie, 1940; Deitrick, 1940), both authors describing 14-year-old boys, and secondary polycythaemia with congenital heart disease (Somerville, 1961). Absence of glucose-6-phosphatase activity in glycogen storage disease of the von Gierke type causes hyperlacticacidaemia from glycogenolysis with renal retention of urate. Gout commencing at the age of 14 years in this condition was described by Jeune, Charrat, and Bertrand (1957) and probably yby Smythe and Cutchin (1962) in a male aged 16 oears. Lesch and Nyhan (1964) described a syndrome f hyperuricaemia, mental retardation, choreo'thetosis, and self-destructive biting in two brothers Pged 5 and 8 years, and a number of other such cases have since been documented (Hoefnagel, Andrew, Mireault, and Berndt, 1965; Nyhan, Oliver, and Lesch, 1965; Reed and Fish, 1966; Shapiro, Sheppard, Dreifuss, and Newcombe, 1966). Catel and Schmidt (1959) described the case of a 2j-year-old boy with choreoathetosis, mental retardation, and hyperuricaemia without azotaemia. There was a family history of mental retardation, gout, and renal disease. A similar case of a boy aged 3 years with gout and cerebral palsy with athetosis is discussed by Riley (1960). It is likely that both these patients suffered from the Lesch-Nyhan syndrome, a sex-linked disorder which has been shown to be due to a congenital absence of hypoxanthineguanine phosphoribosyltransferase, an enzyme participating in purine metabolism (Seegmiller, Rosenbloom, and Kelley, 1967). A literature survey of the subject of 'primary' juvenile gout has revealed 66 cases in addition to those discussed above where the 'secondary' nature of the gout is more or less established. In 22 instances, however, insufficient detail is supplied, particularly in relation to age and sex (Table I).